Jinta Minako, Arai Ayako, Yamamoto Koh, Sakashita Chizuko, Fukuda Tetsuya, Miu Tohru, Koyama Takatoshi, Murakami Naomi, Miura Osamu
Department of Hematology, Tokyo Medical and Dental University.
Rinsho Ketsueki. 2007 Apr;48(4):310-4.
A 19-year-old man was referred to our hospital with pancytopenia and disseminated intravascular coagulation (DIC). Bone marrow aspiration revealed 93.6% of atypical promyelocytes and marked hemophagocytosis by macrophages. The diagnosis of acute promyelocytic leukemia (APL) associated with hemophagocytic syndrome (HPS) was made. As there was no evidence of infection, collagen diseases, or abuse of medicine, his HPS was classified as malignancy-associated HPS (MAHS). The DIC improved after administration of idarubicin and all-trans-retinoic acid (ATRA). On the 11th day, however, DIC and elevation of serum LDH recurred with the appearance of hepatosplenomegaly. Although APL cells had decreased in the bone marrow, hemophagocytes persisted. After administration of dexamethasone and etoposide, DIC and HPS improved, and complete remission of APL was obtained. ATRA was implicated in the aggravation of APL-induced MAHS in the present case.
一名19岁男性因全血细胞减少和弥散性血管内凝血（DIC）被转诊至我院。骨髓穿刺显示93.6%为非典型早幼粒细胞，巨噬细胞有明显的噬血细胞现象。诊断为急性早幼粒细胞白血病（APL）合并噬血细胞综合征（HPS）。由于没有感染、胶原病或药物滥用的证据，他的HPS被归类为恶性肿瘤相关性HPS（MAHS）。给予伊达比星和全反式维甲酸（ATRA）后，DIC有所改善。然而，在第11天，随着肝脾肿大的出现，DIC和血清乳酸脱氢酶（LDH）升高再次出现。虽然骨髓中的APL细胞减少，但噬血细胞持续存在。给予地塞米松和依托泊苷后，DIC和HPS得到改善，APL获得完全缓解。在本病例中，ATRA与APL诱导的MAHS加重有关。
